Four cases of umbilical cord hemangioma are presented and analyzed with all previously reported cases. Umbilical cord hemangiomas consist of an angiomatous nodule containing and encompassed by edema and myxomatous degeneration of Wharton's jelly, often cystic. The angiomatous nodules range from 0.2 to 7 cm in largest dimension, whereas the associated edema may measure up to 15 cm. The tumors tend to occur at the placental end of the cord and presumably arise from one or more of the major umbilical vessels. The angiomatous nodules tend to be sharply demarcated from the surrounding stroma and are invariably cytologically benign. Although cord hemangiomas are strongly associated with fetal morbidity and mortality, a causal relationship has not been definitely established. There is no apparent association between cord hemangiomas and maternal age, race, or gravidity nor does one sex predominate. Unlike placental hemangiomas, cord hemangiomas are not associated with maternal hydramnios. Ultrastructural differences between umbilical cord and placental amnion explain both this differing clinical effect as well as the presence of marked edema and myxomatous degeneration of Wharton's jelly in cord tumors. Speculation concerning an underlying congenital predisposition to vascular neoplasm in these infants is presented.
Three cases of omphalomesenteric (OM) duct cyst within the umbilical cord are presented and analyzed together with 6 previously reported cases. The embryology of the OM duct is reviewed, emphasizing those portions of the duct that may persist beyond early fetal life. Omphalomesenteric duct cysts are located at the proximal (fetal) end of the cord and range from 0.4-6.0 cm in diameter. They appear as cystically dilated "miniature" segments of the gastrointestinal tract and are lined by columnar epithelium resembling that of the gastric, small intestinal, or colonic mucosa; islands of pancreatic tissue may be associated with the cyst lining. The cysts have peripheral angiomatoid vascularity and the surface epithelium of the cord at one site tends to be hyperplastic. These lesions occur more often in males than in females and are unrelated to prematurity or to maternal age, race, or gravidity. They only rarely cause fetal morbidity or mortality but evidence is presented to suggest that they may on occasion be associated with potentially dangerous intraabdominal anomalies of the OM duct.
Involvement of the central nervous system by sinus histiocytosis with massive lymphadenopathy (SHML) is rare. Less than 30 cases have been reported. To the best of our knowledge, we describe the first case of an intramedullary spinal cord lesion which occurred in a 34-year-old black male, causing paraplegia. Laboratory evaluation revealed anemia, increased erythrocyte sedimentation rate, and polyclonal gammopathy. Microscopic examination of the lesion revealed lymphocytes within the cytoplasm of the histiocytic cells, a phenomenon known as emperipolesis. Immunohistochemical stains showed the large eosinophilic histiocytic cells to be positive for S-100 protein and Kp1 antigen. Special stains for fungi and acid-fast organisms were negative. There was no evidence of clonality in the lymphocytic aggregates. Glial fibrillary acidic protein was negative in the lesion, although positive in the surrounding tissue. These features confirmed the diagnosis of extranodal SHML involving the intramedullary spinal cord.
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